[Primary pleural epithelioid hemangioendothelioma, an often-misdiagnosed rare tumor]

Revue des Maladies Respiratoires 2022 April [Link]

P Tossan, E Pichon, F Arbion, D Sizaret, G Simionca, S Marchand-Adam


Introduction: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor that develops in various organs. Primary pleural epithelioid hemangioendothelioma is an exceptional occurrence, with only forty cases reported in the literature. On account of its rarity, pleural EHE is difficult to diagnose. Differential diagnoses such as malignant pleural mesothelioma or lung carcinoma are often initially suspected.

Methods: We herein describe the case of a 52-year-old man presenting with a primary pleural epithelioid hemangioendothelioma revealed by thoracic pain and having evolved for three months. Having reviewed the literature, we consider the clinical presentation and diagnosis modalities of this rare tumor.

Results: After an initial diagnosis of lung carcinoma, an ultrasound-guided biopsy was performed, confirming the diagnosis of pleural EHE in our patient. Ours is the first case of pleural EHE to be diagnosed with ultrasound-guided echography. Presentation of pleural EHE is often clinically and radiologically nonspecific. Most diagnoses are obtained by thoracoscopy, which allows for targeted biopsies and evacuation of pleural effusion.

Conclusion: The diagnostic process for this rare tumor must be rigorous. Ultrasound-guided biopsy may be considered, provided that the lesions are accessible.